Journal
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 21, Issue 13, Pages -Publisher
MDPI
DOI: 10.3390/ijms21134776
Keywords
systemic sclerosis; fibrosis; extracellular matrix; matricellular proteins; myofibroblasts; biomarkers; therapeutics
Funding
- Kidney Research Scientist Core Education and National Training (KRESCENT) Program New Investigator Award [KRES180003]
- Kidney Foundation of Canada
- Canadian Society of Nephrology
- Canadian Institutes of Health Research
- Canadian Institutes of Health Research [MOP-428250]
- Hopital Maisonneuve-Rosemont Foundation
- KRESCENT Infrastructure Support [KRES180004]
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Systemic sclerosis is a rare chronic heterogenous disease that involves inflammation and vasculopathy, and converges in end-stage development of multisystem tissue fibrosis. The loss of tight spatial distribution and temporal expression of proteins in the extracellular matrix (ECM) leads to progressive organ stiffening, which is a hallmark of fibrotic disease. A group of nonstructural matrix proteins, known as matricellular proteins (MCPs) are implicated in dysregulated processes that drive fibrosis such as ECM remodeling and various cellular behaviors. Accordingly, MCPs have been described in the context of fibrosis in sclerosis (SSc) as predictive disease biomarkers and regulators of ECM synthesis, with promising therapeutic potential. In this present review, an informative summary of major MCPs is presented highlighting their clear correlations to SSc- fibrosis.
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